title: Japanese variant of multicentric castleman's disease associated with serositis and thrombocytopenia--a report of two cases: is TAFRO syndrome (Castleman- Kojima disease) a distinct clinicopathological entity?
abstract: Multicentric Castleman's disease (MCD) is a polyclonal lymphoproliferative disorder that manifests as marked hyper-γ-globulinemia, severe inflammation, anemia, and thrombocytosis. Recently, Takai et al. reported a new disease concept, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Furthermore, Kojima et al. reported Japanese MCD cases with effusion and thrombocytopenia (Castleman-Kojima disease). Here, we report two cases of MCD associated with marked pleural effusion, ascites, and thrombocytopenia, and discuss the independence of the TAFRO syndrome (Castleman-Kojima disease). Case 1: A 57-year-old woman had fever, anemia, anasarca, and some small cervical lymphadenopathy. Although she had been administered steroid therapy, and full-coverage antibiotics, her general condition, including fever, systemic inflammation, and anasarca, deteriorated steadily. We administered chemotherapy [CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisolone) regimen], but despite a transient improvement, she died due to septic shock. Case 2: A 73-year-old man with a history of aplastic anemia and remission presented with fever, severe inflammation, and anasarca. Prednisolone was administered (15 mg daily), and his hyperinflammation once improved. Nevertheless, his general condition, including pleural effusion and ascites, worsened, and C-reactive protein and interleukin-6 levels showed marked increases. The patient died due to multiorgan failure. Cases of TAFRO syndrome (Castleman-Kojima disease) are still rare. Therefore, it is necessary to conduct multicenter clinical surveys including similar cases, such as ours, to reach a consensus regarding diagnostic criteria, therapeutic strategy, and pathophysiological etiology for this syndrome.
authors.affiliation: Hematology and Immunology Medicine Kanazawa Medical University, Ishikawa, Japan. yasum@kanazawa-med.ac.jp
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Masaki
authors.forename: Akio
authors.initials: A
authors.lastname: Nakajima
authors.forename: Haruka
authors.initials: H
authors.lastname: Iwao
authors.forename: Nozomu
authors.initials: N
authors.lastname: Kurose
authors.forename: Tomomi
authors.initials: T
authors.lastname: Sato
authors.forename: Takuji
authors.initials: T
authors.lastname: Nakamura
authors.forename: Miyuki
authors.initials: M
authors.lastname: Miki
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Sakai
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kawanami
authors.forename: Toshioki
authors.initials: T
authors.lastname: Sawaki
authors.forename: Yoshimasa
authors.initials: Y
authors.lastname: Fujita
authors.forename: Masao
authors.initials: M
authors.lastname: Tanaka
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Fukushima
authors.forename: Toshiro
authors.initials: T
authors.lastname: Okazaki
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
mesh_terms: D000368:Aged; D044466:Asian People; D005871:Castleman Disease / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012700:Serositis / Q000473:pathology* / Q000628:therapy; D013921:Thrombocytopenia / Q000473:pathology* / Q000628:therapy